Labs
This vasculopathy can give you both a positive PR3 and MPO
What is levamisole vascuiltis
fun fact: can also cause pancytopenia
The ADVOCATE trial proved that the use of this drug allowed for sustained remission with significantly lower doses and duration of steroids for ANCA vasculitis
What is avacopan?
Per ACR 2021 vasculitis guidelines, what is recommended against in severe GPA/MPO with DAH
What is plasma exchagne?
This is the mechanism of action of avacopan
What is a C5a inhibitor?
For patients with GCA who have critical or flow-limiting involvement of the vertebral or carotid arteries, the ACR conditionally recommend adding this medication
What is aspirin?
Recommendation: For patients with GCA who have critical or flow-limiting involvement of the vertebral or carotid arteries, we conditionally recommend adding aspirin.
This is the antibody associated with hypocomplementemic urticarial vasculitis
What is Anti C1q?
The pexivas didn't show improvement in these outcomes with the use of plasma exchange
What is ESRD and death?
For patients with non-severe GPA this drug is recommended for maintenance of remission over AZA or MMF
what is methotrexate?
Recommendation: For patients with active, nonsevere GPA, we conditionally recommend initiating treatment with methotrexate and GCs over trimethoprim/sulfamethoxazole and GCs.
This should be offered to a young patient prior to initiation of treatment with cyclophosphamide
What is egg retrieval or cryopreservation?
The new ACR large vessel vasculitis guidelines recommend this modality for diagnosis of temporal arteritis
What is TA bx?
Recommendation: For patients with suspected GCA, we conditionally recommend temporal artery biopsy over temporal artery ultrasound for establishing a diagnosis of GCA.
EGPA patient will have a positive ANCA 40% of the time. If a patient is ANCA positive, this outcome is more likely
What is renal disease?
What trial showed that there was improvement in renal function in patients with severe renal dysfunction from AAV but no improvements in Death
What is the MEPEX trial?
2007
Plasma exchange increased the rate of renal recovery in ANCA-associated systemic vasculitis that presented with renal failure when compared with intravenous methylprednisolone. Patient survival and severe adverse event rates were similar in both groups.
Per guidelines, it is not recommended to add this medication to maintenance remission therapies in GPA
What is Bactrim?
Recommendation: In patients with GPA whose disease has entered remission, we conditionally recommend against adding trimethoprim/sulfamethoxazole to other therapies (e.g., rituximab, azathioprine, methotrexate, etc.) for the purpose of remission maintenance.
PAN can be associated with Hep B, in these cases what is the first line of treatment?
What is treat Hep B?
then steroids, can move on to other DMARDs if that doesn't work
For patients with severe GPA/MPA whose disease has entered remission after treatment with cyclophosphamide or rituximab, the ACR conditionally recommends what treatment for maintenance?
What is RTX?
Recommendation: For patients with severe GPA/MPA whose disease has entered remission after treatment with cyclophosphamide or rituximab, we conditionally recommend treatment with rituximab over methotrexate or azathioprine for remission maintenance.
This marker is small vessel vasculitis is associated with the lowest risk of relapse
What is MPO?
PR3 has a high risk of relapse
This was the renal cutoff for the PEXVIAS study
Cr >4
GPA/MPA patients refractory to induction therapy should have this added to current therapy
What is IVIG?
Recommendation: For patients with GPA/MPA that is refractory to remission induction therapy, we conditionally recommend adding IVIG to current therapy
for this indication, plasma exchange is still recommended in the guidelines for ANCA vasculitis
What is concomitant ANCA vasculitis and anti GBM?
"Plasma exchange remains advisable in patients with GPA or MPA who also have anti–glomerular basement membrane disease. "
This antibody is present in the active phase of thromboangiitis obliterans
What is anti endothelial antibody?
The French vasculitis group conducted a study on maintenance in AAV called MAINRITSAN. These two drugs were compared
What is rituximab and azathioprine?
More patients with ANCA-associated vasculitides had sustained remission at month 28 with rituximab than with azathioprine. (Funded by the French Ministry of Health; MAINRITSAN ClinicalTrials.gov number, NCT00748644; EudraCT number, 2008-002846-51.)
For patients with active, nonsevere GPA, the ACR recommend initiating treatment with this over cyclophosphamide or rituximab.
What is methotrexate?
For patients with active, nonsevere GPA, we conditionally recommend initiating treatment with methotrexate over cyclophosphamide or rituximab.
ACR large vessel vasculitis guidelines define this as low dose of steroids
What is prednione <10mg/day
Low-dose oral GCs Prednisone ≤10 mg/day or equivalent